My Story
I was diagnosed with Myelofibrosis in July of 2005. A bit of a shock I must say, but my haematologist said not to worry too much as it was a very slow progressing disorder and it was very likely that something else would get me before myelofibrosis did. I probably had decades to go before that might happen. OK, lets get on with life the best I can then.
Well, things don’t always go to plan, do they? In November 2010 I have been transfusion dependent for about two years. I have a spleen so big that my wife calls it the twins because of the size of my belly and a transfusion lasts about 3 weeks if I’m lucky. So they top me up and when my haemoglobin gets back down t 90 g/L, in goes another 3 units.
My white cells are all down too, except for the lymphocytes for some reason, which have remained normal. Neutrophils are now about 0.3 so I should be prone to every bug around, but I don’t seem to get sick any more often than I ever did. And my platelets are pretty well normal too, so I’m not a bleeder. So I carry on with a reasonably normal, if not very physical life while I wait for the research teams to come up with the goods. I know there are some very promising initial results being published but, hey guys, hurry up – I don’t know how long I’ve got! Joking aside, it does look like 2011 will be the year of the first approved treatment for myelofibrosis, something that I am looking forward to.
I have become used to being tired most of the time, and put up with feeling sick and/or uncomfortable with a belly that looks like I’m nearly ready to deliver. I sleep sitting up in bed mostly now days because I don’t breath properly lying down. But I still love to take the dog for a walk most days. She’s getting older so we don’t walk as fast or as far as we used to but we do our half an hour a day, which is more than a lot of people.
There is a saying about people with myelofibrosis which goes something like this; Most days you feel sick and tired, but on the days you don’t, you think about how sick and tired you are of being sick and tired.
Sure, it gets me down sometimes, that’s human nature. But you can focus on how badly off you are, or you can focus on what you can still achieve in your life. So I try to stay positive because I still have some goals I want to reach. So follow me, we’ve got unfinished business.
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Hi Alan,
I like your website.. I have Polycythemia was dx in 1998. I had a very large spleen to until December 2005 when it spontaneously rupture on a Sandringham train to Flinders St. Ended up with a splenectomy.. I wanted to ask you about making a link between our websites. Unfortunately the MPD-Oz site was hacked by russian spammers and it is down at the moment but I have to re do the pages so I will be looking for new links and I was hopeing to add your site to the mix/
Ken Young
MPD-Oz
(Melbourne based – 0408 341 795)
Hi Alan,
I am 51 years old and have had PV for 15 years now and was diagnosed with gout 30 years ago. Took copius amounts of Indocid and other gout meds for years before anyone told me of the long term problems this could cause. Then my spleen was swelling and the doctors said I had PV. Put me on Hydrea and said I would be OK. In July 2010 my spleen split and I was in hospital for 10 days while they made up their minds to take out my spleen or not. It’s still in there albiet battered and bruised with 3 splits in it. Last week had a bone marrow biopsy from the hip (ouch) to see if I have MPD, they think I have and if so want to put me on the JAK2 inhibitor. Do you know anthing of this, (success, failure, side effects) if so please answer me as I’m a tad nervious. I get the results this Friday 20/8/10.
Cheers
Thanks
TonyB
Hi Tony,
You sure have had a rough time of it lately. Removing the spleen is generally only done as a last resort. I was talking to my hematologist just yesterday about this and he was saying that when it was fairly common to do this it caused more problems than leaving it in, and the risks associated with removing the spleen were too great to do it without it being absolutely necessary for the patients survival. So your doctors are quite right to try and save it (and you).
Ouch is right, not the most pleasant thing to have a bone marrow biopsy. But at least it gives you doctors a better idea of where you are in the disease. It will give them a better idea of where to go next. If you have the chance to get on a program to try one of the JAK 1/2 inhibitors, grab it. I wish I could. It won’t cure you, but if it works on you it will certainly make you feel better by reducing the constitutional symptoms such as fatigue, reduce your spleen by up to 50% and there is a good chance that your blood counts will improve as well. The results so far seem to be fairly consistent with these inhibitor drugs in that they seem to help about 75% of people in the drug trials which is quite incredible. There are about a dozen drug companies trialing versions of JAK 1/2 inhibitors so I hope I can hang on until I can get my hands on one which works for me. But if you get the chance, go for it.
I hope I have been helpful, if you need more information, watch the videos from the guys at the Mayo Clinic in the Myelofibrosis section. They are really good value and they keep updating their info by releasing new videos as the drug trial and other treatments progress. So do keep visiting here from time to time to check for updates.
Good luck and my best wishes,
Alan Mead
Thank you, Alan – I would be happy to contribute to your website – is there a post box address I could send to. I cannot risk sending online.
Kate – 26/11/2010
All good wishes Alan, I often forward your interesting and excellent posts to our 3,000 subscriber MPD list here in the States. Robert
i wish you the best of luck and there is medicine out there to reduce the spleen, my dr. at Corneill NY Columbia Presbyterian Dr. Eric Feldman (top in the field) 212 746-6736. He can help you live a long, happy life with feeling like you used to by all the new meds out there.
stacey hayes
mahlerstacey@aol.com
dx 01 et, mf – HU, plavix – blood clot in the colon, feeling great, its been 10 years!
Hi ALan, I read your story with interest. There aren’t too many of us with MF as there are with some other diseases. As of this month I will have had it for 16 years now. I wasn’t diagnosed until June of 2009.
How do i know i had it you ask? Well the blood counts and I had had a BMB many years ago. For some reason the dr and pathologist both dropped the ball. Knowing what i know now I can clearly see the teardrop shaped rbcs and the fibrotoic material. After dg my dr started me on hydroxy urea. At first it worked great!
Go my spleen and liver down smaller. But then he kept raising the dosage and things just kept getting worse.
Turned out I was allergic to the HU and it accelerated the MF tremendously. Finally got off it in March 2010.
Radiation of spleen in April< May 2010. 2nd mistake. It killed the marrow in my spine, ribs and left pelvis leaving me transfusion dependent. Very quickly I developed antibodies to platelets so now can't recieve them.
Just started on revlimid on CHristmas eve. If I can get past the count lowering stage I should be able to hang in there another year or two,. Who knows? WBC 4.6, hemoglobin 7-8, platelets 6-8 not 60 to 80 but 6-8.
Drs amazed I can walk about and function but I'm a tough not so old bird. 53.
Good luck. I wish you all the best.
Karl
Hi Alan,
I was eventualy diagnosed with Polycythemia Vera in 1977 after bone marrow biopisy. Venasection weekly to reduce the haematocrit from 66 down to around 40 and remained ok for over 12 months, only problem with FBC was high platelet count. Took a trip around Australia by caravan 98/99 for seven months. Sometime in 99 started on Hydrea and about threemonthly venasection. Increased Hydrea to try to contol high platelet count, resulted in Anemia and platelet count still high. Change of specialist and another try with Hydrea and same result. Change of medication to Anagrelide to control platelets, worked ok for about 5 years, then had to be stopped as causing diahorea. Put back on Hydrea and platelets remained ok. Venasection about every 4 months.
Had monthly blood test in May 2009 and fairly dramatic drop in Haemoglin Count 110? anothe blood test next week and Hg below 100,GP arranged transfusion and Haematology Specialist requested to bags of packed red cells. This continued with two more transfusions of 2 bags of packed red cells over next 6 weeks.
Bone marrow biopsy carried out and and advise was Polycythemia Vera JAK-2 mutation positive ,
Transforming to Mylofibrosis – Bone Marrow June, 2009.
I was then put on subcutaneous injections of Aranesp 80mg weekly, but increased Hg to 131 and was reduced to biweekly.
The Hg is to be kept between 110/120 an not to exceed 120 because of the danger of stroke.
Now on weekly blood tests and weekly injections of Aranesp either 20 or 40mg depending on Hg couint to keep Hg withinnlimitof 110/120.
Not a great way to be not an excess of energy but it is better looking at the grass than the roots. The worst part is I still have the Polycythemia itch after a shower and I suffer from heat rash if I get to energetic or the humidity gets too high.
Have been watching the Incyte tests and receive mail direct from Incyte on the subject, my spleen is only slightly enlarged so have not been concidered for the Comfort 1 program. This program has been operating in all states of Australia since about September 2009. Check with you Haemaetology Specialist.
Mr. Mead,
I stumbled upon your web-site while researching Myelofibrosis. I commend you for your openness and honesty regarding your illness. And I admire your determination regarding treatment. My husband was diagnosed with Myelofibrosis after a blood test showed his hemoglobin was at 4.4. He required a transfusion and had a CT scan that showed a spleen the size of a basketball. He had a bone marrow biopsy that confirmed the diagnosis.
The reason I decided to write to you is to encourage all of your readers to get these tests done ASAP if there is any question that they may have this. The Doctors have been trying to get my husband to have a CT scan done for years. He had refused to go. Because of the fear of finding out what the enlarged mass in his abdomin was or because it caused him no issues or pain, well, your guess is as good as mine. The trip to the emergency room that finally got the tests done was only because he had no strength to fight me.
Though there is no cure at this time, as you know, there are medications and procedures to keep it in control and allow you to live a reasonably normal, productive life.
My husband is 81 yrs old and there is no way of knowing how long he has had this, undiagnosed. He also has diabetes, renel failure, and has suffered from bouts of gout for many years. His spleen is now so large that it has wrapped itself around and adhered to any other organ in its way. He has back and shoulder pain and is so tired all the time he spends half of the day in bed. The doctor had been giving him Aranesp to help keep his hemoglobin up and it has been working. Now, as of last week, they tell me his insurance will only cover 80% of this, which leaves us with at least a $600 bill a month (depending on the dose he requires). I have been researching insurance and prescription plans everyday, until my head is spinning and I just can’t absorb any more information. One of the roadblocks with that is his age and pre-existing conditions. I am determined to keep trying though. I have heard of a JAK 2 trial locally that I am going to inquire about.
But again, I am only writing this in the hope that it will encourage anyone else that may have this to not delay in getting the tests done and treatment started. Please don’t wait, like my husband did. The end result may not change, but the quality of your life and that of your loved ones may be impacted by your decision.
Thanks again for this chance to not only voice this, but to see that we are not alone in this. God Bless you and all of the people who shared their stories on here. I pray there may one day be a cure for this, sooner rather than later.
Debi Drill
Hi there!
I was diagnosed with ET Essential Thrombocythemia 12 years ago when i was 31. I had no symptoms other than a high platelet count. My doctor told me my ET may stay the same and cause no concern for years and it would not shorten my life. He also told me it could change to one of the other myloproliferative (not how you spell it) disorders, but then again it may not. Well, 4 years ago it did change after the birth of my daughter to Myelofibrosis. I haven’t had any symptoms but my bone marrow aspiration showed there was a lot of scar tissue (fibrosis). I do get very tired, but that’s probably because I’m a Mum to a full on 5 year old.
I have blood tests every 3 months. My hemo advised when I start to sweat at night and get anemic they will then think of the right treatment for me. The transplant team at my hospital are currently doing a world search for a donor bone marrow as no one in my family was a match. Once they find someone, they will put that bone marrow on ice till I need it, I may never need it!
This is a horrible thing to have hanging over your head, but I try not to focus on it and live life to the fullest. I pray to God for His continual healing in my life and believe it won’t get any worse.
Having a positive outlook with this is important, but there are days I do have moments of worry especially when you have a young family.
Praying for a cure!
Alan –
as a newly diagnosed myelofibrosisian, i am deeply grateful for the posts you have written. it’s like i’m just learning to ride a bike and there you are behind me, holding the seat and shouting words of encouragement. right now, two days before my 78th birthday, i am a mixture of hope, wonder, shock, acceptance, optimism, itching, and eagerness to learn.
i’ve had 12 units of blood transfused into me since december of last year, and i feel wonderful all the time — except after i exert myself, and then i feel wonderful though very out of breath. only last week was i diagosed. at first, the docs thought my breathlessness was due to a vitamin b-12 deficiency. i loved that diagnosis! but the need for more transfusions caused the docs to look further and that’s when myelofibrosis reared its ugly spelling.
at this point, i exist on hope, new information, sensitive posts, and the love of my wife and daughter. i can’t wait till i get to read good news, post good news, and experience an end to my out-of-breathness and itching. meanwhile, i am seriously blessed with the ability to write books and teach marketing, which is what i write about. what if i were the shortstop for the chicago white sox? my career would be over.
but now, i feel poised at the end of an adventure. i’ll take whatever comes, having crossed out all the entries on my bucket list. still, i am very appreciative of your insights, alan. thank you, dude.
jay
Hi to everyone. My names Amanda and im looking for people in Australia who have this rare blood disorder Polycythemia Vera. My partner is a 60 year man and he has been diagnosed with polycthemia vera. And i am scared, confused and would like to get in contact with anyone in Australia who is suffering the same. I would also so like to know if there is a support foundation in Australia. Thank you to every one.
Hi. Thanks for the great site – only just found you. I live in NW Scotland and like Clyde started with PV which in my case was diagnosed 8 years ago. I am now aged 55. In January this year I had a routine monthly blood count done. The last in December showed a haematocrit of .446 so I expected it would be up and I’d need another venesection. I had got very tired and my spleen (measured at 21cm in July) seemed bigger and I was losing weight. Anyway hct was about .37. Checked again and no mistake. Saw specialist 2 weeks ago – spleen now 25cm got slide done showing various indicators of MF. Awaiting outcome of other tests including bmb. Seeing her again next month. Had about two good weeks but washed out again. Didn’t think it would come so quickly. Here’s hoping the new drugs come soon if it goes this fast! Keep up the good work.
Hi Alan and Simon and all others who have an intrest in Mylofibrotic Diseases.
I have given a previous note about my experience with Polycythemia Vera, which after 12 years, eventualy in 2009 resulted in the “Spent Phase” in that the Bone Marrow is now Fibrotic, and does not produce the required number of red blood cells. Now I am on weekly blood tests and injections of Aranesp(darbepoetin alfa) either 20mg or 40mg, depending on blood Haemeglobin (Hg), to maintain a blood Hg of no less than 110 and not greater than 120.
What I would like to talk about is the “shock” even though I knew that in some time in the future the “Spent Phase” of Polycythemia would happen.This all took place in April, May June 2009.
I was aware of most of the literature that was written about this blood disease, particulary from literature from:- http://mpdinfo.org/faq/faq_pv.html . I guess that after 12 years of Polycythemia and daily medication of Hydrea and Venasection every 3/4 months I had lost sight of the future destination I was heading too.
This was the “Shock Phase” going from Venasection to Blood Transfusion of Packed Red Cells to maitain the Hg instead of having a monthly blood test to check the Haematocrit (Hae) to maintain a blood percentatge of 40/45% to plasma in each blood cell.
How often was it going to happen, how would my body system react, how long could this go on for, how do I tell my family what is happening to me. This for me was not a happy time but a depressing time as to what the future held.
Most important thing to me was that my family were there for me and my GP then proceed to talk of the future and the way to deal with it, one day at a time and there was a long way to go before any major decisions had to be made.
The next turning point was in August/September 2009 when the Haematology Specialist made the suggestion that after 3 Bags of Packed Red Cells, he would like to try Aranesp injections. The injection of Arenesp worked for me even though it has taken around 15 months to be somewhat stable in the range of Hg he has set. It is not perfect but he has suggested I may have another ten years which is very encouraging.
Now I will be able to enjoy my 80th birthday in July, and hopefully soon, playing golf again.
Not everything is fair or perfect in this world but we must all look forward to the future and put our trust in the people who are best able to advise us of what the future holds.
Clyde.
sharon says,
i was diagnosed with polycythemia rubra vera in 2005 commenced on interferon,deralin,omeprazole,plavix
on interferon injections for 10months felt really sick,tired,ill weak just blagh stopped the interferon due to the side effects.
commenced on Hydrea different doses other medication the same,i have a swollen spleen,portal vein thrombosis the blood has found new ways to travel to reach the liver and legs.
varices at the top of the stomach which may burst and bleed.all diagnosed 2005 have managed to work go out travel somes days look good but feel sick.
in december /jan 2011 diagnosed with myeleofibrosis low HB,short of breath,indigestion,bloating,had a repeat bone marrow aspirate now waiting for a bone marrow transplant as things have progressed quickly from 1 illness to another they are worried about changing to acute myeloid leukaemia.
so my siblings have been tested for donor match and poss August it will happen.
i am normally very active never sit down,not looking forward to being stuck in a room,in sterile conditions,losing my hair from the intense chemo.i am 52 have travelled the world love visiting new places and im going to be a great aunt in July.
back on interferon injections to attempt to shrink the spleen,so it improves my chances,have not told many people untill we have a date for procedure, no point in worrying everyone to soon.
well good luck to you all, no 2 are the same ,
sharon
I have had polycythemia vera for 3 years now and have tried very hard to get on a jak2 inhibitor without success. Can anyone help me?
Interesting…I guess this is not a rare disease at all, seems like most cases start with Essential Thrombocystosis(?) I’m in the US, and believe it or not it took awhile to diagnose this condition.
I guess like most Americans we don’t bleed or show pain…SMILE.
UC, Mayo Clinic, Cleveland, and some others have been on what they call the cutting edge of ET studies, but clinicals are underway, some of the drugs you’ve all talked about are used, but unfortunately in the States this disease is not considered worth the time others are, so even though I look extremely healthy, I can actually feel my stem cells trying to fail.
God Bless all of you who can have a stem cell transplant, and the disease does not progress!!!!!!
hi, i was dxd with et, 8 mnths ago, i get seriuos night sweats and bone pain as well as being very tired, just had a bmb and it showed i had mod fibrosis and retculin, is that ealry mf? thank god i found a site in australia,
Hi Julie, if bmb shows fibrosis, looks like your one of us. Up to you haematologist to confirm of course. The other symptoms are indicaters also. Hopefully RUXOLITINIB will be available here soon, but it will probably take a while to get onto the PBS. It sounds strange, but I was so relieved when I was diagnosed (6 years ago now) because it explained everything, especially the tiredness. I hope everything goes well for you with your treatment.
Keep fighting
AM
Hi AM,
Thank you for that advice, thats what i thought, but one dr says yes and the other no, so very very confused, i;ve had some of these symptoms for years, i can write my full report if you wont? I;m on full vile of interferon and 1,000mg of hu a day at the moment, so feeling pretty tired and low, once again thanks
Looking for people in Singapore who have this rare blood disorder Polycythemia Vera. now 36 and has been diagnosed with polycthemia vera 2 month ago. And i am scared, confused and would like to get in contact with anyone in Singapore who is suffering the same. Thank you
Mail me: lesbeng1@hotmail.com
I was diagnosed with ET 4 months ago ….. wondering if anyone has trialled a homeopathic remedy and if so which one.
I was diagnosed PV in 1998 it was about 14 years ago and i was in Hydrea during that time. No limitation in doing activities at all…so don’t worry. But started last year after being treated with Hydrea for such a long time, the disease slowly move to become MF with severe anemia. Last week I was treated in the hospital due to Hb keep dropping eventhough being transsfused with bags of blood. After being treated with hydrocortizone (a kind of steroid) and my hb starting stable and went home. I am in a plan to have blood transfusion every 3 weeks to keep me up. No pain in my body at all….I do hope my hb stay at normal level. Does anyone can give me advissce to keep the hb at normal level?
i have mylofibroisis, i feel i’m pregnant as the spleen is enlarged and does palpitate a lot. i’m 62 had this problem 10 years, i’m on chemo pills, also taking a kidney pill ,i’ve had 8 bouts of radiation. what makes me feel bad is that depends what i eat if it doesn’t agree with me i have pains. i’m a widow my family members think well basically they do not feel, that i should say nothing as they have their own problems. i try to stay away but it’s hard .i went to princess margeret in toronto i ,m not a canditate for a stronger pill . i feel confused but i’m not capable of doing stuff i used to do ,i,m always sleeping . take care lucy
Hi Lucy, My spleen sounds like it’s about the same size as yours. My wfe refers to it as the twins. Only it’s been 9 months many times over and it’s still there. I have no treatment other than transfusions, the drug Jakafi is not available here and my haematologist thinks that most of the treatments available to me are not worth the side effects they cause. It is hard at times, I feel really ill in the evenings when I try do too much during the day, and I don’t talk about it much, as you say, family often doesn’t want to know too much. My wife is very supportive, however, she has her own problems so I do keep how I’m feeling to myself most of the time. The fatigue and tiredness is a real problem but I manage to get a fair bit done most days. I am getting to the point where if I don’t get access to the new drugs soon, I won’t be able to use them due to my worsening blood counts. Anyway, you have found us here and as you can see, there are a lot of other sufferers here, so I hope you can find out more about myelofibrosis. I try to keep it up to date so maybe you will be less confused after going through some of the latest posts. Good luck.
My father inl aw has mylefibrosis and he was on a jack inhibitor 2 trial for about 2 years and he was fine with it he didn’t need any more blood transfusons and he was very active. About 4 weeks ago he startet to feel very tired and he coudn’t do the same activities he was used to. They done the blood tests and about 2 weeks ago toock him of the trial beacuse the plantifs where under 50. The doctor told us then that is not in his bone marow yet.From then on it was down hill, he is just getting more and more tired. Even if he goes and have a blood transfusion i dosent seem to do much. He finds it hard to breath to . We cannot belive its all hapening so quick he is such an active kind of person and now is hard for him to get in to the shower. Does anyone have any ideea what else can we do? The doctor just told us today that he is giving him 3 months… its just hard to belive that is nothing, no other treatment he can try.
The Leukaemia Foundation is currently updating the Understanding Myeloproliferative Neoplasms booklet – and we would love some cosumer feedback on the myelofibrosis sections – if anyone would like to review it for us and offer suggestions on how we could improve it, I’d love to hear from you: asteele@leukaemia.org.au
I have had a high platelet count for 30 years in September of this year. I was dx with mylefibrosis this week and was told I am a good candidate for bone marrow transplant. Anyone had or know someone that has had a transplant. I am 56 yo and probably will not be able to find family with a match. Glad I found this site. Very helpful and encouraging!